Gene Symbol | GM2A |
---|---|
Entrez Gene | 2760 |
Alt Symbol | GM2-AP, SAP-3 |
Species | Human |
Gene Type | protein-coding |
Description | GM2 ganglioside activator |
Other Description | cerebroside sulfate activator protein|ganglioside GM2 activator|shingolipid activator protein 3|sphingolipid activator protein 3 |
Swissprots | D3DQH6 Q14428 Q14426 Q6LBL5 B2R699 P17900 |
Accessions | AAD25741 AAF63788 EAW61680 EAW61681 P17900 AK225978 AK298867 BAG60987 AK312494 BAG35396 BC009273 AAH09273 BM834780 BX473154 DQ891914 ABM82840 DQ895098 ABM86024 L01439 AAA52767 M76477 AAA35907 X16087 CAA34215 X61094 CAA43407 X61095 CAA43408 X62078 CAA43993 NM_000405 NP_000396 NM_001167607 NP_001161079 |
Function | The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity (By similarity). Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta- hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3. {ECO:0000250}. |
Subcellular Location | Lysosome. |
Top Pathways | Lysosome |